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Case Report

Cerebellar Neuroblastoma in 2.5 Years Old Child

Mohammad Pedram1, Majid Vafaie1, Kiavash Fekri1, Sabahat Haghi1, Iran Rashidi2, Chia Pirooti3

Abstract 1. Thalassemia and Neuroblastoma is the third most common malignancy of childhood, after Hemoglobinopathy Research Center, Ahvaz Jondishapur University of and brain tumors. Only 2% of all neuroblastoma occur in the brain. Primary Medical Sciences, Ahvaz, Iran cerebellar neuroblastoma is an specific subset of Primitive Neuroectodermal 2. Dept. of Pathology, Shafa Tumors (PNET). Meduloblastoma is a relatively common and well-established Hospital , Ahvaz Jondishapur entity, consisting of primitive and multipotential cells that may exhibit some University of Medical Sciences, evidence of neuroblastic or gliad differentiation. But cerebellar neuroblastoma Ahvaz, Iran with ultrastractural evidence of significant neuroblastic differentiation is 3. Dept. of Neurosurgery, Golestan extremely rare. We report a rare case of neuroblastoma in the cerebellum. A Hospital, Ahvaz Jondishapur 2.5-year-old Iranian boy presented with vomiting and nausea in the morning and University of Medical Sciences, . CT scan showed a tumor mass in the cerebellum and the report of Ahvaz, Iran radiologist was . Light microscopic assay showed a small cell Corresponding Author: with lobules of densely packed cells (lobulated pattern) and better Mohammad Pedram, MD; differentiated cells. -Specific Enolase was positive. Pathologic diagnosis Professor of Pediatrics Hematology- confirmed the existence of cerebellar neuroblastoma. followed Oncology surgical removal. No relapse occurred 12 months after . Tel: (+98) 611 374 32 85 Email: Keywords: Neuroblastoma; Cerebellum; Chemotherapy [email protected] Please cite this article as: Pedram M, Vafaie M, Fekri K, Haghi S, Rashidi I, Pirooti Ch. Cerebellar Neuroblastoma in 2.5 Years Old Child. Iran J Prev. Received: 2 Jan. 2012 2013; 6(3):174-6. Accepted: 18 Feb. 2012 Iran J Cancer Prev 2013;3:174-6

Introduction Neroblastoma is the most common extracranial site of cerebellum, the tumor maybe classified as solid tumor in children, accounting for 8% to 10% of cerebellar . all childhood [1-4]. When they form a lobulated pattern with cells A tumor mass can occur anywhere along the streaming in a fine fibrillated background, they are sympathetic nerve crest pathway [2]. designated cerebellar neuroblastoma [1, 7, 8]. Therefore, a wide variety of tumor locations and Review of epidemiologic studies and case reports clinical presentations have been described [5] but revealed that there is not a reported case of only 2% of all nerublastoma arise in the brain [6]. cerebellar neuroblastoma as a primary CNS tumor in The most common malignant of childhood Iran [9-11]. Here, we describe a rare case with is Medulloblastoma [1, 2]. Medulloblastoma, cerebellar mass, which pathological diagnosis was medullophithelioma, and neuroblastoma are cerebellar neuroblastoma. designated in the WHO classification as embryonic and cytologically pluripotential tumors. Case Report Microscopically, exhibit highly We report a patient with primary cerebellar variable histological features, most frequently neuroblastoma. A 2.5-year-old boy was admitted to appearing as an infiltrative, extremely our hospital because of nausea and vomiting in the undifferentiated, cellular neoplasm [3, 5]. The cells morning, headache, ataxia, poor balance and have carrot - shaped or ovoid nuclei with course torticulli. The preoperative routine complete blood dark chromatin and scant or no visible cytoplasm. count , clotting profiles , electrolytes , sequential Mitoses and necrosis are highly variable. Homer – multiple analysis for chemistry , and the urine wright rosettes, the core of which is formed or analysis findings were all within the normal limits . A tenuous fibrillated processes, may be present. brain CT scan revealed a well defined hyperdense Exceptionally, in various stages of maturation mass in cerebellar vermis compressing forth ventricle are observed. When matured cells are present at the with obstructive hydrocephaly, compatible with medulloblastoma (Figure 2). A microscopic

Vol 6, No 3, Summer 2013 174 Cerebellar Neuroblastoma in 2.5 Years Old Child suboccipital craniectomy was performed and after During this hospital admission, postoperative systemic complete resection of the tumor, a examinnation which included bone survey, bone ventriculoperitoneal shunt was inserted. marrow aspiration, abdomino-pelvic CT scan and The pathologist reported a composed of small, MRI of the neuroaxis revealed no other evidence of dense and round cells with hyperchromatic nucleus tumor. Also cytologgical examination of cerebrospinal and scanty cytoplasm. The lesion arranged in sheets fluid was normal. After surgery we began and prevascular accumulation numerous foci of small chemotherapy with a combination of , Ara-c, cells with few mitotic figures. There were necrosis and , Hydroxyurea, Lamustin, Procarbazin, hemorrhagia. Also lobulated tumor cells were and Methyl prednisolon (8 at 1 arranged in groups (islands separated by few protocol) every four weeks for 12 courses. Follow up fibrous stroma). Parallel patterns were seen in some examinnation revealed a profound delay in mental foci of tumor cells. Also cells with vesicular nuclei development but brain MRI performed 12 months were seen (Figure 2). These findings established the after surgery revealed no evidence of tumor diagnosis of neuroblastoma. Immuno-Histo-Chemistry recurrence. The patient is presently 3.5 years old (IHC) showed a strong positivity for Neuron-Specific and his chemotherapy is finished and he is stable Enolase (NSE). with no sign of relapse.

Figure 1.This figure shows Figure 2. This figure shows pathological slide brain CT scan at the time of diagnosis. of tumor (H&E staaining).

Discussion researchers have not observed the ultrastructural Neuroblastoma is an enigmatic tumor in its clinical characteristics of neuronal differentiation in and biologic behavior [1]. Emberyonal tumors or medulloblastoma [13]. Yagishita et al. examined a Primitive Neuroectodermal Tumors ( PNET ) are the series of medulloblastomas by histological and most common groups of malignant CNS tumors of immunohistochemical techniques and proposed the childhood , accounting for 20 – 25 % of pediatric hypothesis that meedulloblastoma was a stem – cell CNS tumors [3]. Primitive neuroepithelial tumors of lines, astrocytic, ependymall, neuronal and the CNS, with few or absent foci of cellular oligoddendroglial although "cerebellar differentiation, are called primary cerebral neuroblastoma" could arise from differentiation of a neuroblstoma or medulloblastoma depending on a medulloblastoma along neuroblastic lines [14,15]. supra – or infratentorial location of the mass, Cerebellar "neuroblastomas" are extremely rare respectively [1]. A wide variety of tumors are known [15]. There are a few reports of cerebellar to occur in the cerebellum and many of these are neuroblastoma in the papers. Shuji Nishio et al. referred to as medulloblastoma [12]. (2000) reported a case of cerebellar neuroblastoma Medulloblastoma, accounting for 90% of embryonal in an infant separately. This tumor displays a better tumors, is a cerebellar tumor [4]. Their cells are prognosis than thoose with classical or desmoplastic primitive or poorly differentiated and are medulloblastoma. The optimal treatment for patients considered to be of embryonic origin [13]. Its with this tumor, however, remains uncertain. This differentiating capacity is one of disputes. Many tumor tends to occur in young children particuularly in

Vol 6, No 3, Summer 2013 175 Pedram et al.

infants, and this further makes the treatment 5. Tatis N, Tavuz M, Reis A. primary cerebral problematic [16]. Our patient was treated by total neuroblastoma : a case Treated with adjuvant removal of the tumor followed by chemotherapy. He chemotherapy and radiotherapy . The Turkish Journal of was survived one year after operation without pediatrics. 2004; 46: 182-5. recurrence. 6. Polat P, Kuntarci M, Eren S, Suma S. Primary cerebral neuroblastoma. JBR-BTR. 2005; 66: 148- 9. 7. Powers JM, Horoupian D. Disease of the nervous Acknowledgment system and sensory organs : Anderson's pathology. Ten th We appreciate all staff in oncology ward of edition. Mosby. 1996; 26: 93 -2831. Shafa Hospital, neurosurgery ward in Golestan 8. Brandis A, Heyer R, Hori A, Walter GF. Hospital, Dr. Iran Rashidi in pathological laboratory cerebellarneuroblastoma :An important differential of Shafa Hospital and Dr. Kaveh jaseb in Shafa diagnosis from cerebellar neuroblastoma and medulloblastoma. Neuropediatrics 1997; 28(4): 235- 8. oncology ward. 9. Alimohamadi SM, Ghodsi SM, Ketabchi SE. Epidemiologic patterns of primary brain tumors in Iran. Conflict of Interest Asian Pac J Cancer Prev. 2008 Apr-Jun; 9(2):361-2. There is no conflict of interest in this study. 10. Mehrazin M, Rahmati H,Yavari P. Epidemiology of primary intracranial tumors in Iran, 1978-2003. Asian Pac J Cancer Prev. 2006 Apr-Jun; 7(2):283-8. Authors' Contribution 11. Miabi Z, Midia M, Midia R. EVALUATION OF Majid Vafaei wrote the case report. Kiavash Fekri PEDIATRIC INTRACRANIAL NEOPLASM IN TABRIZ, IRAN. and Sabahat Haghi contributed to the literature Acta Medica Iranica. 2007; 45(6):458-65. review, discussion and patient follow up. Iran Rashidi 12. Yungshin W, Laufer H, Lee YC , Aftalion B, Hirano contributed to the pathological findings. Chia Pirooti A, Zimmerman HM. Fine structure of a cerebellar drafted the neurosurgical findings of the case. neuroblastoma. Acta neuropath (Berl).1978; 42: 11-13. Mohammad Pedram performed supervision and final 13. Yagishita S, Itoh Y, Chiba Y, Yamashita T, edit of the paper. Nakazima F, Kuwabara T. Cerebellar neuroblastoma. Alight and ultrastractural study. Acta neuropath. 1980; 50(2): 139- 142. References 14. Yagishita S, Itoh Y, Chiba Y, Kuwana N. 1. Garrett MB, John MM. Neuroblastoma :Pizzo PA , Morphological investigations on cerebellar Poplack DG , Principles and practice of pediatric "neuroblastoma" group. Acta neuropath. 1982; oncology , 5th edition . Philadelphia: Lippincot – Raven ; 56(1): 22-8. 2006 . 933- 62. 15. Pearl GS, Tuke Y. cerebellar " neuroblastoma " 2. Lanzkowsky P. Nerublastoma: Manual of pediatric nosology as it relates to medulloblastoma . Cancer.1981; hematology and oncology. 5th ed. 2004, 530-47. 47: 772 -9. 3. Muralium. Chintagumpala. Malignant brain tumors. 16. Nishio Sh, Inamura T, Morioka T. cerebellar Oski's pediatrics. Principles and practice . 4 th edition. neuroblastoma in an infant . Clinical neurology and Lippincott Williams and Wilkins ; 2006 . 1771- 5. neurosurgery. 2000; 102 (1): 52 -7. 4. Johnn LA. Neuroblastoma. Kliengman , Behrman , Jenson , stanton, Nelson text book of pediatrics , 18 th edition ,Saunders Elsevier company ; 2007 . 2137- 140.

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